Table 1

IL-1 blockade in NLR-related disease

Autoinflammatory disease

Gene mutated

Activator

IL-1 antagonist used

References


Muckle-Wells syndrome (MWS)

NALP3 (CIAS1/NLRP3)

Anakinra

[61-68]

Rilonacept

[79]

Familial cold autoinflammatory syndrome (FCAS, FCU)

NALP3 (CIAS1/NLRP3)

Anakinra

[65,69-73]

Rilonacept

[79]

Chronic infantile neurological cutaneous and articular syndrome/neonatal onset multisystem inflammatory disease (CINCA/NOMID) [43]

NALP3 (CIAS1/NLRP3)

Anakinra

[69-71]

Rilonacept

[79]

Familial Mediteranean fever (FMF) [44]

MEFV

Anakinra

[165-168]

Pyogenic arthritis, pyoderma gangrenosum and acne syndrome (PAPA)

PSTPIP1

Anakinra

[169,170]

Vitiligo [86]

NALP1 (NLRP1)

?

Gout

MSU

Anakinra

[58,59]

Pseudogout

CPPD

Anakinra

[60]

Hyperimmunoglobulin D syndrome (HIDS)

Mevalonate kinase

Anakinra

[166,171]

Systemic-onset juvenile idiopathic arthritis (SoJIA)

Anakinra

Rilonacept

[79]

Behçet's disease (BD)

IL-1β polymorphism

Anakinra

[172-174]

Schnitzler's syndrome

Anakinra

[77]

Blau syndrome (BS)/early onset sarcoidosis

NOD2

Anakinra

[160]

Crohn's disease (CD)

NOD2

Anakinra (Ineffective)

[158]

Ulcerative colitis (UC) [52,53]

MEFV (in a proportion of patients)

?

Other diseases

Hydatidiform mole [87]

NALP7 (NOD12, NLRP7)

?

Hypertension [54]

NALP3

?

Asthma [175]

NOD1

?


IL, interleukin; NLR, NOD-like receptor.

Mathews et al. Arthritis Research & Therapy 2008 10:228   doi:10.1186/ar2525